Minimally Conjoined Omphalopagus Twins with a Body Stalk Anomaly

Abstract

Introduction This report will discuss a case of minimally conjoined omphalopagus twins (MCOTs) with a body stalk anomaly (BSA).

Case Report We experienced monochorionic diamniotic (MD) twins born at 31 weeks. One infant was suspicious of BSA before birth, and another infant was normal. But normal infant had anal atresia with small intestine which was inserted behind the umbilicus. Twins had very short common umbilicus and infant with BSA had intestinal conjunction, two appendixes at the site of the colon, and a blind-ending colon. We diagnosed MCOTs.

Discussion On the basis of the Spencer hypothesis, the etiology of MCOTs was that MD twins shared a yolk sac. However, this could not explain the presence of a BSA. It is necessary to consider the possible reasons for a singleton BSA. In addition, intestinal fusion occurred unequally in this case, although two appendixes were found in the same place, which might have occurred because of the balanced fusion.

Keywords

body stalk anomaly - intestinal conjunction - minimally conjoined omphalopagus twins

• References

• 1 Karnak I, Sanlialp I, Ekinci S, Senocak ME. Minimally conjoined omphalopagi: emphasis on embryogenesis and possibility of emergency separation. Turk J Pediatr 2008; 50 (5) 503-508
• 2 Kaufman MH. The embryology of conjoined twins. Childs Nerv Syst 2004; 20 (8-9) 508-525
• 3 Mann L, Ferguson-Smith MA, Desai M, Gibson AA, Raine PA. Prenatal assessment of anterior abdominal wall defects and their prognosis. Prenat Diagn 1984; 4 (6) 427-435
• 4 Kapur RP, Jack RM, Siebert JR. Diamniotic placentation associated with omphalopagus conjoined twins: implications for a contemporary model of conjoined twinning. Am J Med Genet 1994; 52 (2) 188-195
• 5 Koltuksuz U, Eskicioğlu S, Mehmetoğlu F. Minimally conjoined omphalopagus twinning: a case report. Eur J Pediatr Surg 1998; 8 (6) 368-370
• 6 Poenaru D, Uroz-Tristan J, Leclerc S , et al. Minimally conjoined omphalopagi: a consistent spectrum of anomalies. J Pediatr Surg 1994; 29 (9) 1236-1238
• 7 Tihtonen K, Lagerstedt A, Kähkönen M, Kirkinen P. Diamniotic omphalopagus conjoined twins in a diamniotic pregnancy. Fetal Diagn Ther 2009; 25 (3) 343-345
• 8 Weston PJ, Ives EJ, Honore RL, Lees GM, Sinclair DB, Schiff D. Monochorionic diamniotic minimally conjoined twins: a case report. Am J Med Genet 1990; 37 (4) 558-561
• 9 Collins J. Notes on a case of twins united by an abdominal sac. Trans Pathol Soc Philadelphia 1885 1887; 13: 276-280
• 10 Spencer R. Minimally united ischiopagus twins: infraumbilical union with cloacal anomalies. J Pediatr Surg 1996; 31 (11) 1538-1545
• 11 Walton JM, Gillis DA, Giacomantonio JM, Hayashi AH, Lau HY. Emergency separation of conjoined twins. J Pediatr Surg 1991; 26 (11) 1337-1340
• 12 Shih JC, Shyu MK, Hwa SL , et al. Concordant body stalk anomaly in monozygotic twinning—early embryo cleavage disorder. Prenat Diagn 1996; 16 (5) 467-470
• 13 Daskalakis G, Sebire NJ, Jurkovic D, Snijders RJ, Nicolaides KH. Body stalk anomaly at 10-14 weeks of gestation. Ultrasound Obstet Gynecol 1997; 10 (6) 416-418
• 14 Streeter GL. Focal deficiencies in fetal tissues and their relation to intra-uterine amputation. Contrib Embryol 1930; 22: 1-44
• 15 Van Allen MI, Curry C, Gallagher L. Limb body wall complex: I. Pathogenesis. Am J Med Genet 1987; 28 (3) 529-548
• 16 Giacoia GP. Body stalk anomaly: congenital absence of the umbilical cord. Obstet Gynecol 1992; 80 (3 Pt 2) 527-529